Ocular abnormalities in patients with beta-thalassemia: a prospective study
DOI:
https://doi.org/10.18203/2349-3259.ijct20210078Keywords:
Beta-thalassemia, Ocular Abnormality, Blood Transfusion, FerritinAbstract
Background: Beta thalassemia is severe genetic blood disorder caused by mutation in the gene encoding for the beta chains of haemoglobin and individuals with beta thalassemia require life-long red blood cell transfusions to survive which leads to hemosiderosis and affects all the organs in the body including eyes. The study aims to study ocular manifestations in these children and its correlation with age and serum ferritin levels.
Methods: A descriptive cross-sectional study was done in government medical college Jammu in 2014-2015 on 67 BTM children. Children below 3 years of age and with pre-existing ocular abnormalities, infections were excluded from the study. Ocular examination was done for any major eye abnormality, congenital malformations or trauma. Retinal examination was done using direct and indirect ophthalmoscope, changes were recorded.
Results: 47.76% of children belonging to the group: 5-10 years, while only 5.97%, i.e., 4 in number pertained to the age group: >15 years. Lens opacity was seen in 29.85% of children. 31 children showed changes in retinal activity, while decreased visual acuity was also observed in 17 children. Iris was significantly affected in children with serum ferritin >2500 ng/ml. Even greater lenticular changes and opacities were observed in children with higher serum ferritin levels (>4500 ng/ml).
Conclusions: As life expectancy for beta-thalassemia patients extends, regular monitoring of the progression of disease and symptoms to detect early changes in their ocular system is recommended, to achieve a better quality of life for this patient group.
References
Vichinsky EP. changing pattern of thalasemia worldwide. Ann N Y Acad Sci. 2005;1054:18-24.
Galanello R. Origa R Beta-thalasemia. Orphanet J Rare Dis. 2010;5:11.
Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World health organ. 2008;86:480-7.
Olivieri NF, Brittenham GM, Mclaren CE, Templeton DM, Cameron RG, Mc Clelland RA et al. long-term safety and effectiveness of iron chelation therapy with deferiprone for thalasemia major. N Engl J Med. 1998;339:417-23.
Song D, Dunaief JL. Retinal iron hemostasis in health and disease. Front aging neurosci. 2013:5:24.
Gaba A, Souza PD. Chandra J, Narayan S, Sen S. Ocular changes in beta thalasemia. Ann opthalmol. 1998;30:357-60.
Verma JC. Burden of genetic orders in India. Indian J Pediatrics. 2000:67:12.
Jafari R, Heydarian S, Karami H, Shektaei MM, Dailami KN, Amiri AA, et al. Ocular abnormalities in multi-transfused beta-thalassemia patients. Indian J Ophthalmol. 2015;63:710-5.
Gartaganis SP, Zoumbos N, Koliopoulos JX, Mela EK. Contrast sensitivity function in patients with beta-thalassemia major. Acta Ophthalmol Scand. 2000;78:512-5.
Cao A, Galanello R. Beta-thalassemia. Genet Med. 2010;12:61-76.
Davies SC, Marcus RE, Hungerford JL, Miller MH, Arden GB, Huehns ER. Ocular toxicity of high-dose intravenous desferrioxamine. Lancet (London, England). 1983;2:181-4.
Orton RB, De Veber LL, Sulh HM. Ocular and auditory toxicity of long-term, high dose subcutaneous deferoxamine therapy. Can J Ophthalmol. 1985;20:153-6.
Taher A, Bashshur Z, Shamseddeen W, Adbulnour REE, Aoun E, Koussa S, et al. Ocular findings among Thalassemia patients. Am J Ophthalmol. 2006;142(4);704-5.
Sorcinelli R, Sitzia A, Figus A, Lai ME. Ocular findings in beta thalassemia. Metabolic Paediatric Systemic Ophthalmol. 1989;13(1).
Taneja R, Malik P, Sharma M, Agarwal MC. Multiple transfused thalassemia major: Ocular manifestations in a hospital based population. Indian J Ophthalmol. 2010;58:125 30.
Gibson JM, Chaudhuri PR, Rosenthal AR. Angioid streaks in a case of beta thalassaemia major. Br J Ophthalmol. 1983;67:29 31.